Limbic encephalitis A rare presentation of the small-cell lung carcinoma

Two patients with an acute organic brain syndrome and accompanying neurological symptoms are described. Extensive work up showed that both patients suffered from small-cell lung cancer. Cerebral metastases were absent. Following chemotherapy and radiotherapy to the primary tumor one of the two patients showed a complete remission of psychiatric symptoms for one year. A paraneoplastic origin of this syndrome, in the literature known as limbic encephalitis, is postulated. The exact cause of this syndrome is yet unknown. Recent research reveals data indicating an immunological pathogenesis. The major clinical importance of this (neuro)-psychiatric syndrome is that its appearance may serve as a warning sign for an occult malignancy; furthermore, effective treatment of the primary malignancy can reverse the encephalitis. Thus antitumor therapy can result in a prolonged survival and considerably improved quality of life.     

Mixed carcinomatous neuropathy in patients with lung cancer and lymphoma

A clinical, electrophysiological and histological study of the peripheral nerve was performed on 62 patients with lung cancer and 30 patients with lymphoma. There was a mild peripheral neuropathy (MPN) in 17.7% of patients with lung cancer and in 10% of those with lymphoma. MPN was related to the degree of weight loss. Histological studies of 5 patients with MPN demonstrated a loss of large myelinated fibers in all patients, axonal degeneration in 2 and remyelination in 3 patients. Electromyographic and histological findings indicate a primary axonal neuropathy with remyelination, probably secondary to axonal damage. 2 patients with lymphoma developed a severe neuropathy that remitted in 1 patient. The main pathological abnormality was segmental demyelination in both cases. Immunofluorescence studies were negative in all cases with mild or severe neuropathy.     

Paraneoplastic coma and acquired central alveolar hypoventilation as a manifestation of brainstem encephalitis in a patient with ANNA-1 antibody and small-cell lung cancer

Introduction

Paraneoplastic neurological disorders are a well recognized complication of malignancy.

Methods

A case report to expand the currently described clinical manifestations of type 1 antineuronal antibody (ANNA-1)-associated paraneoplastic encephalomyelitis to include coma.

Results

We present an unusual case of fluctuating coma and rapid fulminant progression to acute respiratory failure from central alveolar hypoventilation caused by ANNA-1 paraneoplastic encephalomyelitis associated with small-cell lung carcinoma. Paraneoplastic infiltration of the brainstem and cerebellum, including respiratory and arousal centers, was documented on autopsy.

Conclusions

Paraneoplastic encephalomyelitis should be considered as a possible cause of coma and central alveolar hypoventilation.     

The "pulvinar sign" in a case of paraneoplastic limbic encephalitis associated with non-Hodgkin's lymphoma

This paper reports a 59 year old woman with paraneoplastic limbic encephalitis associated with diffuse large B cell lymphoma. Her brain magnetic resonance imaging scan showed bilateral posterior thalamic hyperintensities, similar to the "pulvinar sign". Her symptoms included progressive psychiatric disturbance and resembled the initial symptoms of variant Creutzfeldt-Jakob disease (vCJD). Clinicians should consider this treatable disorder in the differential diagnosis of vCJD.     

The carcinomatous neuromyopathy of oat cell lung cancer

A prospective controlled clinical-neurophysiological-pathological study of 71 patients with oat cell carcinoma of the lung revealed no increased incidence of peripheral neuropathy at the initial stages of illness. All patients developed neuropathy by the time they had lost 15% of their body weight, but the neuropathy was less severe than in 20 age-matched alcoholic patients with an equal degree of weight loss. The weight loss and peripheral neuropathy progressed with atrophy of type II (adenosine triphosphatase-positive) muscle fibers out of proportion to the patient's loss of body weight. By 40% body weight loss, all the patients had moderate symmetrical peripheral neuropathy, 6 had proximal brachial or lumbosacral plexus metastases, and 9 had distal pressure palsies. Mononeuritis multiplex developed in only 1 patient, who had diabetes mellitus. Two patients developed Eaton-Lambert syndrome, which resolved in 1 when chemotherapy controlled the systemic tumor, with no protein in the tumor postmortem which could produce the characteristic electromyographic findings of the syndrome.     

Electromyographic and nerve conduction findings in a patient with neuromyotonia, normocalcemic tetany and small-cell lung cancer

A 57-year-old man with neuromyotonia and normocalcemic tetany as probable paraneoplastic symptoms of small-cell lung cancer was examined neurophysiologically. In EMG, spontaneous action potential generation was demonstrated in peripheral motor axons. There were also time-locked high-frequency discharges after some voluntarily activated motor unit potentials. After electrical stimulation of motor axons, the M-re-sponse as well as spinal F-response or H-reflex was often followed by a sequence of oscillating potentials which could last several hundred msec. There was no evident peripheral polyneuropathy. Tetany subsided when carbamazepine therapy was started. The only striking abnormality in extensive laboratory studies was an increase in the plasma noradrenaline value.     

Autoantibody against platelet glycoprotein IIb/IIIa in a patient with non-Hodgkin's lymphoma

An autoantibody to platelet glycoprotein (GP) II b/III a was produced in a 38 year-old woman who had a previous history of the malignant lymphoma of the stomach. The aggregations of the patient's platelets showed losses of the primary waves in response to ADP and epinephrine and marked hypoaggregation in response to collagen, while agglutination by ristocetin was normal. Crossed immuno-electrophoresis (CIE) of her platelets solubilized by 1% Triton X-100 revealed an abnormal biphasic precipitate line of GP II b/III a complex. Nine months later, she developed severe thrombocytopenia along with a relapse of the lymphoma in the cervical lymph nodes. The patient's IgG, which was collected during her thrombocytopenic period and purified, inhibited ADP-, epinephrine- and collagen-induced aggregations of normal platelets. In CIE, the 125I-labelled IgG of the patient, inserted into the intermediate gel, was incorporated into the precipitation line of the GP II b/III a complex of normal platelets. Radiation treatment to the cervical lymph nodes dramatically normalized both the function and the count of the patient's platelets. From these findings, it is suggested that an autoantibody to the GP II b and/or III a was produced by the lymphoma cells.     

Autoantibodies in paraneoplastic syndromes associated with small-cell lung cancer

An antineuronal autoantibody has been identified in serum from 14 patients, 8 women and 6 men, with small-cell lung carcinoma (SCLC) and a neurologic disorder. Neurologic symptoms began prior to diagnosis of the SCLC in 12 patients. The dominant neurologic disorder was a subacute sensory neuronopathy (SSN) in eight patients, SSN plus lower motor neuron weakness (2 patients), SSN plus autonomic neuropathy (1 patient), cerebellar ataxia (1 patient), myelopathy (1 patient), and multifocal nervous system disease (encephalomyelitis) in one patient. The presence of the same autoantibody in patients with SSN, encephalomyelitis, and autonomic neuropathy suggests that these diseases are different manifestations of the same nosologic process. With one exception, treatment of the tumor, immunosuppressive drugs, and plasmapheresis did not influence the course of the neurologic illness. The autoantibody was not identified in sera from more than 400 controls subjects, including patients with SSN associated with other tumors, SSN without malignancy, other paraneoplastic syndromes, and SCLC without neurologic symptoms. The autoantibody is a highly specific marker of the paraneoplastic syndromes associated with SCLC and its detection in a patient not known to have cancer should prompt a careful search for SCLC.     

Paraneoplastic movement disorder in a patient with non-Hodgkin's lymphoma and CRMP-5 autoantibody.

The paraneoplastic autoantibody, collapsin response-mediator protein (CRMP)-5 immunoglobulin G (IgG), is specific for neuronal cytoplasmic CRMP-5, and is usually associated with small-cell lung carcinoma or thymoma. We report on details of a movement disorder that followed anti-B-cell therapy in a patient with lymphoma, and was accompanied by CRMP-5 IgG.     

Triphasic sharp waves in a patient with carcinomatous meningitis

Subacute encephalopathy with an electroencephalographic (EEG) pattern of generalized periodic triphasic sharp waves occurred in an 80-year-old woman with autopsy proven carcinomatous meningitis. Meningeal carcinomatosis should be included within the differential (clinical) diagnosis of such an EEG pattern.     

The hot cross bun sign in a patient with encephalitis

The ‘hot cross’ bun (HCB) sign refers to pontine cruciform hyperintensity on T2 weighted magnetic resonance image (MRI) which is frequently seen in multiple system atrophy and spinocerebellar ataxia types 2 and 3. We describe a 3?years old boy of encephalitis and his MRI image showed HCB sign in the pontine. After immunosuppressive treatment and followed up 14?months, he got a good outcome and the HCB sign narrowed nearly disappeared.     

Direct evidence for systemic fibrinogenolysis in a patient with metastatic prostatic cancer.

Although the possible occurrence of systemic fibrinogenolysis has been suggested in patients with metastasising prostatic cancer (MPC), direct evidence is lacking. We report on a patient with MPC whose laboratory data were consistent with hyperfibrinolysis: marked decrease of alpha 2-antiplasmin (AP) level (less than 50% of normal), increase of plasmin-alpha 2-antiplasmin complex, D-fragment of fibrin and fibrinogen degradation products [FDP(D)] and cross-linked fibrin degradation products (XDP). The patient neither showed laboratory nor clinical evidence for consumption coagulopathy except for a slight increase in thrombin-antithrombin III complex level. Immunoblotting of the patient's serum using an anti-fibrinogen antibody revealed the presence of a 250 kDa protein in addition to DD fragments. Following reduction of this protein by 2-mercaptoethanol after extraction from SDS-PAGE gel, gamma-chain of fibrinogen (47 kDa) was found by immunoblotting using a monoclonal antibody recognising a 86-302 residue of the gamma-remnant of fibrinogen. Moreover, the 250 kDa protein did not bind to Sepharose 4B to which a monoclonal antibody recognising the N-terminus of fragment D was conjugated. These findings indicated that this protein was not fragment DY, but rather fibrinogen fragment X. With the retraction of the prostatic tumour by an effective therapy, the patient's AP level increased gradually. When the plasma AP level rose to 60% of normal, the fragment X was no longer detectable. These findings suggested that systemic fibrinogenolysis occurred in the patient with MPC only when AP levels were markedly decreased.     

The encoding capacity of a patient with amnesia due to encephalitis

A patient with amnesia due to encephalitis was examined in an effort to identify the factors that might contribute to his memory disturbances. It was discovered that this patient had the ability to analyze incoming verbal information on a level comparable to that achieved by normals. Furthermore, this ability enabled him to retain a limited amount of information for a brief interval, but, as soon as further information was introduced, it appeared to displace completely the older information in his working memory. As a consequence, information never seemed to be consolidated into permanent, long-term, memory. Several attempts to increase the amount of information that could be consolidated (e.g. through use of visual imagery, verbal mediation, etc.) were attempted with promising, yet limited, results.